Mutation Screening of the ALS2 Gene in Sporadic and Familial Amyotrophic Lateral Sclerosis

Abstract
AMYOTROPHIC LATERAL sclerosis (ALS) is an adult-onset neurodegenerative disorder characterized by the death of motor neurons in the cortex, brainstem, and spinal cord, resulting in progressive paralysis and death, usually within 3 to 5 years of symptom onset.1,2 Most ALS cases are sporadic, without any family history. Approximately 15% to 20% of cases are familial, and within these lie several distinct forms of the disease.